Review Question - QID 5553

This child has symbrachydatyly of the left hand. This is associated with Poland syndrome which is characterized by unilateral absence of the sternocostal head of the pectoralis major and chest wall hypoplasia on the ipsilateral side, as seen in Figure C

Poland syndrome is a congenital disorder which includes unilateral chest wall hypoplasia, absence of the sternocostal head of pectoralis major, hypoplasia of the hand and forearm, symbrachydactyly, and breast or nipple aplasia. It is 3 times more common in males and 75% of cases involve the right side

Kramer et al. retrospectively compared syndactyly release in Poland syndrome vs idiopathic syndactyly. They found significant differences in function between operated and normal hands in Poland syndrome, but not in idiopathic syndactyly. They concluded that functional deficits in hands affected by Poland syndrome is attributable to more than the syndactyly alone, whereas hands with idiopathic syndactyly are likely to have little postoperative functional deficit.

Van Heest described congenital disorders of the hand and upper extremity. She characterized Poland syndrome to include ipsilateral hand anomalies such as brachydactyly, syndactyly, or ectrodactyly. Other associated anomalies include hypoplastic scapula or ribs, or shoulder girdle muscles (e.g. latissimus dorsi), scoliosis and renal anomalies.

Figure A shows synbrachydactyly of the left hand. In Poland syndrome, the chest wall deformity is on the ipsilateral side as the synbrachydactyly.

Incorrect Answers:
Answer 1: Figure B shows pectus excavatum. This is associated with Marfan syndrome but not with Poland syndrome.
Answer 3: Figure D shows a normal CT chest (bone window).
Answer 4: Figure E shows pectus carinatum. This is not associated with Poland syndrome.
Answer 5: Figure F shows a right rib osteochondroma. This is not associated with Poland syndrome.